How is a PAPVC defect corrected?

Question

Hi doctor,

I am an 18-year-old girl who was diagnosed with a PAPVC (partial anomalous pulmonary venous connection) defect in the left upper pulmonary vein seven months ago. I have been experiencing symptoms for quite a while, such as poor circulation and exercise intolerance, which seem to worsen even as I gradually try to build up my endurance. My current cardiologist does not support the idea of fixing it, even though my MRI shows that despite having only one vein, the blood flow ratio from one side to the other is noticeably borderline.

I have an appointment with an electrophysiologist because I seem to be tachycardic quite often, even though my pulse used to be in the 70s. Additionally, I have been having episodes that they think could be related to an arrhythmia, possibly linked to the defect.

Please help.

Answer 1

Hi,

Welcome to icliniq.com.

Have you experienced frequent respiratory tract infections, such as pneumonia, since childhood? Please upload your MRI (magnetic resonance imaging) and echocardiogram reports. Is your hemoglobin level normal? Typically, a single vein shunt is not considered significant, and the severity of the shunt can be assessed on an echocardiogram as well. Surgery for small defects is generally not considered unless the shunt ratio is greater than 2:1, as surgery has no impact on survival rates.

Therefore, if the reports do not indicate any damage to the heart, surgery may not be necessary. However, you should be given a trial of low-dose diuretics, such as Torsemide at 5 to 10 mg, and monitor your response in terms of exercise tolerance. If your hemoglobin is low, it should be corrected, as low levels can cause these symptoms. You should try to exercise and gradually increase your activity without focusing on the defect.

Yes, a cardiac arrhythmia may be associated with the defect. Since you are experiencing frequent palpitations, you should undergo Holter monitoring, which involves 24-hour ambulatory ECG (electrocardiogram) monitoring. This will help detect the heart rhythm during palpitations and confirm whether an arrhythmia is present. Regarding treatment, you may be prescribed beta-blockers, such as Metoprolol, after we rule out or confirm the diagnosis related to your palpitations.

Thanks and regards.

Answer 2

Hi,

Welcome back to icliniq.com.

Is your hemoglobin level and chest X-ray normal? This is to ensure that your symptoms are related only to your heart condition and not to an alternative cause. Now, considering the shunt fraction of 2:0.9, which is equivalent to 2.2:1, and the fact that the right side of your heart is enlarged, as you mentioned, this becomes an indication for surgery. Therefore, I believe surgery should be performed in your case.

Thanks and regards.